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Stahl ear deformity associated with Finlay-Marks syndrome.

A 6-year-old child with Finlay-Marks syndrome presented to our clinic for evaluation of bilateral congenital auricular deformities (figure). Examination of the pinnae revealed bilateral Stahl ear deformity. Both auricles were prominent, with the superior crus of each ear extending at a right angle from the antihelix. As a result, the helical fold demonstrated posterior over-projection at the point where the aberrant superior crus intersected with the helical rim. The lobule and tragus were hypoplastic. Surgical correction was offered hut declined.

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Auricular anomalies can be categorized embryologically as malformational or deformational. (1) Malformational auricular anomalies are those caused during early embryologic development, as the auricle develops between the 6th and 12th gesrational week. (2,3) Such anomalies include anotia, microtia, cleft ears, polyotia, preauricular tags or sinuses, and absent or supernumerary cartilage bars--all of which exhibit severe deficiencies or significant aberrations of the major auricular landmarks. (1)

Deformational anomalies are those occurring from extrinsic forces on an otherwise normally shaped ear in utero or postnatally. Deformation can be caused by abnormal insertion of auricular muscles, muscle imbalance, or abnormal positioning of the ear. Thus, anomalies such as prominent ear, crinkled ear, Stahl ear, and lop ear are included in this category. (1,4)

Several theories exist on the etiology of Stahl ear, including developmental error during the 3rd embryologic month as the helix and scaphoid fossa mature, deforming pressure in utero, abnormal prechondral growth, failed embryologic regression, and abnormal transverse intrinsic muscle orientation. (2,5) Yotsuyanagi et al found an abnormal transverse intrinsic auricular muscle as the culprit causing Stahl ear. (6) The frequent bilateral nature of this deformity does suggest that the responsible deformational force occurs by more than mere happenstance. Bilateral Stahl ear may arise from an embryologic directive, perhaps resulting in abnormal symmetric placement of transverse intrinsic auricular muscles.

While most cases of Stahl ear occur without other congenital anomalies and without hereditary disposition, (4,7) the physician should he aware of possible concomitant craniofacial, audiologic, renal, and other multiple congenital anomalies. In our patient, this ear malformation was associated with Finlay-Marks syndrome, a rare autosomal dominant condition consisting of congenital abnormalities of the scalp, ears, and nipples. (8-10) Otherwise, in the absence of further physical exam findings, the presence of Stahl ear alone does not require additional testing. (1,6)

Surgical approaches to correct Stahl ear are not straightforward. As the long axis of the abnormal crus is often far from parallel to the normal long axis of the antihelix, simple Mustarde suture methods may not be viable. Many published surgical strategies involve either aggressive cartilage reshaping or repositioning techniques. These include cartilage shaving, scoring incisions, cartilage grafting, and excision and repositioning of the aberrant superior crus. (4,5,7,11) If identified during the first 3 months to 1 year of life, auricular molding with thermoplastic splints or surgical taping could be attempted. (12)

References

(1.) Porter CI, Tan ST. Congenital auricular anomalies: Topographic anatomy, embryology, classification, and treatment strategies. Plast Reconstr Surg 2005;115 (6): 1701-12.

(2.) Hunter AG, Yotsuyanagi T. The external ear: More attention to detail may aid syndrome diagnosis and contribute answers to embryological questions. Am J Med Genet A 2005;135(3):237-50.

(3.) LaFrentz, JR. Unusual anomaly of the external ear. Ear Nose Throat J 2004;83(8):534.

(4.) Aki FE, Kaimoto CL, Katayama ML, et al. Correction of Stahrs Ear. Aesthetic Plast Surg 2000;24(5):382-5.

(5.) A1-Qattan MM, Hashem FK. An alternative approach for correction of Stahl's ear. Ann Plast Surg 2004;52(1): 105-8.

(6.) Yotsuyanagi T, Nihei Y, Sinmyo Y, Sawada Y. Stahl's ear caused by an abnormal intrinsic auricular muscle. Plast Reconstr Surg 1999; 103(1):171-4.

(7.) Ferraro GA, Perrotta A, Rossano F, D'Andrea E Stahl syndrome in clinical practice. Aesthetic Plast Surg 2006;30(3):348-349; discussion 350.

(8.) Finlay AY, Marks R. An hereditary syndrome of lumpy scalp, odd ears and rudimentary nipples. Br J Dermatol 1978;99(4):423-30.

(9.) Edwards MJ, McDonald D, Moore P, Rae J. Scalp-ear-nipple syndrome: Additional manifestations. Am J Med Genet 1994;50 (3):247-50.

(10.) Taniai H, Chen H, Ursin S. Finlay-Marks Syndrome: Another sporadic case and additional manifestations. Pediatr Int 2004;46 (3):353-5.

(11.) Ono I, Gunji H, Tateshita T. An operation for Stahl's ear. Br J Plast Surg 1996;49(8):564-7.

(12.) Tan ST, Abramson DE, MacDonald DM, Mulliken JB. Molding therapy for infants with deformational auricular anomalies. Ann Plast Surg 1997;38(3):263-8.

Young S. Paik, MD; C.W. David Chang, MD

From the Department of Otolaryngology-Head and Neck Surgery, University of Missouri-Columbia School of Medicine, Columbia, Missouri.
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Title Annotation:FACIAL PLASTIC SURGERY CLINIC
Author:Paik, Young S.; Chang, David
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Geographic Code:1USA
Date:Jun 1, 2010
Words:773
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